Hereditary Renal Cancer
Over the past 5 years there have been dramatic developments in the understanding of hereditary renal cancers. von Hippel-Lindau disease is now understood to be associated with clear cell carcinoma, and other associations have also become known: hereditary papillary renal cancer is associated with type 1 papillary renal cancer, and hereditary leiomyoma renal cell carcinoma with type 2 papillary renal cancer. Birt-Hogg-Dubé syndrome and familial renal oncocytoma are associated with chromophobe carcinoma and oncocytomas, although other histologic tumor types have been found in Birt-Hogg-Dubé syndrome. Medullary carcinoma of kidney is associated with the sickle cell trait.
Although the genes associated with these tumors have been discovered, the exact mechanisms by which they cause renal cancer remain to be elucidated. It is quite likely that other genes also are involved in this process. Using VHL disease as an example, research is now underway on using mutant pVHL or excess HIF for diagnostic and therapeutic purposes. Understanding the mechanisms that lead to cancer may open new avenues of opportunity for drug development. This improved knowledge of the biogenetic pathways used to form tumors will affect the development of new therapeutic techniques for treating both hereditary and nonhereditary forms of renal cancer.
KeywordsRenal Cell Carcinoma Renal Tumor Renal Cancer Clear Cell Carcinoma Sickle Cell Trait
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